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Cystic Fibrosis

Cystic Fibrosis. Diyana Zainal Lorena Ferchaud. What is Cystic Fibrosis?. Inherited mutation in the protein: Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) > 1500 mutations possible, not all resulting in cystic fibrosis Affects lungs and digestive system

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Cystic Fibrosis

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  1. Cystic Fibrosis Diyana Zainal Lorena Ferchaud

  2. What is Cystic Fibrosis? • Inherited mutation in the protein: • Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) • > 1500 mutations possible, not all resulting in cystic fibrosis • Affects lungs and digestive system • Body produces thick and sticky mucus due to electrolyte imbalance • Occurs in the first decade of life, average lifespan is 31 years (O’Riordan, 2009) (O’Sulivan, 2009) (Stallings, 2008)(Farrell, 2008)

  3. Symptoms of Cystic Fibrosis http://www.nhlbi.nih.gov/health/dci/images/cysticfibrosis01.jpg

  4. Who is at risk for Cystic Fibrosis? • 1 in every 2,500 live births worldwide; 1 in 31 Americans are carriers • Conception between 2 carriers will result in • 25% chance their child will have Cystic Fibrosis • 50% chance the child will be a carrier • 25% the child will be a non-carrier • Most will be diagnosed by age 3, 10% of new cases are diagnosed at 18 years or older (Nelms, 2007)

  5. Prevalence of Cystic Fibrosis (O’Sullivan 2009)

  6. http://2.bp.blogspot.com/_AH0As01Tvu8/SamqR-uzuyI/AAAAAAAAAAU/sT2bBu5Nb5s/S1600-R/inheritance.gifhttp://2.bp.blogspot.com/_AH0As01Tvu8/SamqR-uzuyI/AAAAAAAAAAU/sT2bBu5Nb5s/S1600-R/inheritance.gif

  7. Diagnostic Process (Farrell, 2008)

  8. Sweat Chloride Test • Tested only at accredited CF care centers • Stimulate sweat glands • Collect onto filter paper • Analyze chloride concentration • Chloride concentrations of >60mmol/L is diagnostic of the disease, but not the severity. (O’Sullivan, 2009) (Ferrell, 2008) Sodium and Chloride are collected

  9. Are All Newborns Tested? • Not all states require testing at birth. • 40 states today have testing, all states by 2010 • Patients can be pancreatic sufficient or insufficient • Pancreatic sufficient patients have a milder case and are not diagnosed until later in life. • Age of onset of symptoms are variable • 2 CF mutations vs. 1 CF mutation • Some infants die in utero, some require surgery at birth for meconium ileus • In medieval times midwives would instruct mothers to lick their newborns (Hudson 2002) (Farrell 2008)

  10. Medical Nutrition Therapy • 110% to 200% of energy needs with high calorie, high fat diet • To support weight maintenance • Strong correlation between nutritional status and pulmonary function • Recommended to maintain BMI at or above 50th percentile • Nutritional supplements • Pancreatic enzyme • Fat soluble vitamins • Babies who are breastfed confer lifelong benefits • 500 to 2,500 units lipase per kilogram body weight per meal (O’Sullivan, 2009) ( Stallings, 2008)

  11. Cystic Fibrosis Treatment Cystic Fibrosis Physiotherapy

  12. University HospitalCystic Fibrosis Case Study Patient: Lily Johnson 14 years old Female Diagnosed with Cystic Fibrosis at 6 months

  13. Lily Johnson • Age: 14 years old • Sex: Female • Participates in ballet, jazz and cross country running. Typically runs 3-5 miles, 5-6 times a week.

  14. Chief Complaint Patient reports trouble breathing and her inhaler is not helping. She believes she may have caught a cold from working at a camp for the past two weeks. Her doctor assumes it is pneumonia due to the fact she is hospitalized approximately once a year for pneumonia.

  15. Medical History • Diagnosed at 6 months • Hospitalized for respiratory infections approximately 1x per year, but otherwise, uneventful disease course • Treatment: High frequency chest compression vest, 1 hour twice daily • High frequency chest compression video

  16. Nutrition History • Appetite fine until the last few days • Enjoys fruits, vegetables and most foods but doesn’t drink milk • Mom and grandmother cook healthy, but not always home to eat meals • Fried foods like French fries and pizza causes diarrhea • Realizes the need for a healthy diet, and willing to make changes

  17. 24 hr recall/usual food intake 24 hour recall • Breakfast - nothing • Lunch – 2 oz hotdog and bun, 1½ c macaroni and cheese (boxed mix, 2% milk) • Dinner – 5 oz Salisbury steak with ¼ c gravy, few bites green beans, 1 roll + 2 T margarine, 2 c grape juice Usual intake • Breakfast – usually nothing • Lunch - 3 T peanut butter or 2 oz ham and 2 oz Swiss cheese sandwich, 2-3 oz chips, orange or other fruit, water • Dinner – 5-6 oz lean meat, grilled or baked, 1-2 c green salad, ¼ c ranch dressing, 1 c starch side dish, water

  18. Medical Diagnosis • Acute pneumonia confirmed by chest x-ray and sputum cultures

  19. Physical exam • Vitals • 99.1 F • BP 114/60mm Hg • Anthropometric • Ht 5’5” • Wt 102 lbs (25th+ percentile) • UBW: 110-115lbs (3 months ago) • Chest/lungs: • percussion hyperresonant • rhonchi • rales decreased breath sounds

  20. Stature for age • 75th percentile Weight for age • 25th-50th percentile

  21. Lab Values • WBC - 13 (normal 4.8 –11.8) indicating infection • HGB - 11.5 (12-15) • HCT - 33 (37-47) • Ferritin - 19 (20-120) • Transferrin – 219 (250-380) • Magnesium – 1.6 (1.8-3) • HbA1C – 6.3 (3.9-5.2) family history of T2DM, possibly cystic fibrosis-related diabetes All other labs within normal range iron deficient anemia (due to medication)

  22. Cystic FibrosisRelated Diabetes (CFRD) • Common and serious problem, in pancreatic-insufficient individuals • Close correlation with deterioration in lung function and loss of weight • Oral Glucose Tolerance Testing (OGTT) is the most sensitive method to detect CFRD

  23. Food Preferences and Allergies • The patient is an active 14 year old and will eat almost anything, but doesn’t like milk. • She tries to avoid fried foods due to resulting diarrhea. • She has no known allergies.

  24. Nutrition Care Process • Cystic Fibrosis patients need 110 - 200% more kcals than normal to maintain homeostasis. • Harris Benedict Equation BEE = 655 + (9.6 x 46kg) + (1.8 x 165.1cm) - (4.7 x 14y/o)=1328 kcals BEE x IF x AF =1328 kcals x 1.5 x 1.5 = 2988 kcals/day Macronutrients: Protein – 15% - 113g = 452 kcals Fat – 35% - 117g = 1053 kcals CHO – 50% - 374 g = 1496 kcals 3001 kcals (Stallings 2008)

  25. Actual vs Recommended Intake 1775 kcals 80g fat 65g protein 198g CHO (actual) 3001 kcals 117g fat 113g protein 374g CHO (recommended)

  26. Potential Diet-Drug Interactions • Pancrease (1-3 caps after meals) • Pancreatic enzyme replacement • Prevacid (20mg daily) • May decrease abs of Fe and B12 • Humabid (1/2 tablet every 12 hours) • Expectorant (such as Mucinex or Robitussion) • Multivitamin • Proventil PRN • Bronchodilator (Pagna 2007, Progna 2008)

  27. Various intake formats • Main format - oral intake • A relatively healthy teenager hospitalized with pneumonia would not be a good candidate for enteral nutrition supplementation • She will need to consume meals with high nutrient density several times a day until her health (and weight) is restored.

  28. PES Statement 1) Food and nutrition related knowledge deficit (NB-1.1) related to time lapse since last education and change in energy expenditure as evidenced by inconsistent and unregulated use of prescribed medications. 2) Involuntary weight loss (NC – 3.2) related to increase in exercise and failure to increase caloric intake as evidenced by a 10% weight loss over a 3 month period.

  29. Scope of Practice

  30. Sample Menu:44% CHO, 19% protein and 38% fat • Breakfast • Honey nut cheerios and whole milk • Sourdough bread • Banana • Lunch • Ham and cheese sandwich • Romaine salad and ranch dressing • Orange • Dinner • Steak • Mashed potatoes • Baby carrots • Dinner roll • Caesar salad with dressing • Mid morning snack • Cheese crackers • Afternoon Snack • Peanut butter and jelly sandwich • Yogurt • Supper • Grapes • Frozen yogurt

  31. Outcome goals • Short term: no further weight loss • Long term: maintain BMI at or above the 50th percentile

  32. Action oriented goals • Patient can demonstrate knowledge of diet plan appropriate for a highly active cystic fibrosis patient

  33. Monitor and evaluate • Track weight • Monitor if patient is following an increased energy intake diet • WBC • HGB • HCT • Ferritin • Transferrin • Magnesium • HbA1C

  34. Follow up • RD visits every 3 months • Referral to diabetes educator, if needed

  35. References • Britto C., Oliveira C., Gomes M., Cunha A. Bras Pneumol. 2009;35(5):409-414 • Farrell P, et al. The Journal of Pediatrics. 2008; 153:S4-S14 • Hudsen, V. Leaven. 2002;38:5:99-102. • Moran A. Pediatric Diabetes 2009:10(Suppl.12):43–50. • Nelms, M. Nutrition Therapy and Pathophysiology. 2007:731-737 • O'Sullivan B., Freedman S. The Lancet. 373: 9678, 30 May 2009-5 June 2009, Pages 1891-1904 • O’Riordan S, Robinson P, Donaghue K, Moran A. Pediatric Diabetes 2009: 10(Suppl. 12): 43–50 • Pagana, Kathleen Deska, and Timothy J. Pagana. Mosby’s Diagnostic and Laboratory Test Reference. Saint Louis: Mosby Elsevier, 2007. • Pronsky, Zaneta M. comp. Food and Medication Interactions. Birchrunville: Food- Medication Interactions. 2008. • Stallings V, Stark L, Robinson K, et al. J Am Diet Assoc. 2008;108:832-839.

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